Richard M A, Grob J J, Philip N, Rey J, Chamson A, Mege J L, Andrac L, Faure F, Basseres N, Bonerandi J J
Service de Dermatologie, Hôpital Sainte-Marguerite, Marseille, France.
Dermatology. 1998;197(2):127-31. doi: 10.1159/000017983.
Stiff-skin syndrome (SSS) is a rare cutaneous syndrome characterized by stony-hard skin and limitation of joint mobility. Its cause is still unknown.
Biological investigations were performed in a new case of SSS.
Collagen production and DNA biosynthesis were studied from fibroblast culture. Proinflammatory cytokines (TNF-alpha, IL-6 and TGF-beta2) were measured in the patient's serum. Results were compared with pathological findings.
Collagen production and DNA biosynthesis were normal whereas the level of circulating cytokines was high. Histological examination of the skin showed mild fibrosis in the dermis whereas the fascia was not thickened.
Our clinical and biological findings suggest that in this case, cutaneous changes may be related to an inflammatory process rather than to a primary fibroblast defect or a fascial abnormality as previously hypothesized.
硬皮综合征（SSS）是一种罕见的皮肤综合征，其特征为皮肤坚硬如石以及关节活动受限。其病因仍不明。
对一例新的硬皮综合征病例进行生物学研究。
从成纤维细胞培养物中研究胶原蛋白生成和DNA生物合成。检测患者血清中的促炎细胞因子（肿瘤坏死因子-α、白细胞介素-6和转化生长因子-β2）。将结果与病理发现进行比较。
胶原蛋白生成和DNA生物合成正常，而循环细胞因子水平较高。皮肤组织学检查显示真皮轻度纤维化，而筋膜未增厚。
我们的临床和生物学发现表明，在该病例中，皮肤变化可能与炎症过程有关，而非如先前假设的与原发性成纤维细胞缺陷或筋膜异常有关。
